There Are No Perfect Numbers - Making Decisions

In mathematics, there are perfect numbers.

The first one happens to be 6.

 But in aortic disease, there are no perfect numbers.

Just 2 millimeters more

How big is 2 millimeters? It is the thickness of a US dollar coin. Looking at it, one wonders how such a small distance could make so much difference. For one man, it was the difference between having surgery or not. His aorta was 5.8 cm. The doctors told him surgery is done at 6.0 cm. The difference - just 2 mm - the thickness of a coin.

When "6" was the Number
For Louie Lopez, this tiny length became a distance too far, a point never reached. He was in the hospital, talking with the surgeon, when his aorta tore and fully ruptured. Massive bleeding, massive damage. Rushed to surgery, he had no chance. The year was 1995. Yes, "6" was "the number" for aortic aneurysm surgery back then.

Not long ago, Terry Lopez talked about that time, almost 20 years ago, when she lost her husband, Louie. "They were waiting for just 2 mm more. Just 2 mm....surely, 20 years later they can do better than that."

Reading papers, watching medical conference videos from thought leaders in thoracic aortic disease, I marvel at the paucity of answers, the preponderance of uncertainty still, as they continue to debate aneurysm size and the timing of surgery. There is still more unknown than known. Aortic disease remains a more than worthy opponent for those who do battle with it.

The Numbers

Physicians look for numbers to guide them in making decisions about aortic disease. For aneurysms, the most prominent number is the size (diameter) of the aneurysm. There is a great deal of debate about the number, the size when surgery should be done. Some argue to protect from what can be serious complications of surgery, urging that patients wait for the aorta to grow larger. Others promote earlier surgery, at smaller sizes, to protect from life-threatening, deadly events.

The following paper from 2013 is an example of the debate, giving pro and con perspectives from physicians around the globe, Germany, the US, and Australia:

"Aortic Surgery for Ascending Aortic Aneurysms Under 5.0 cm in Diameter in the Presence of Bicuspid Aortic Valve",  authored by Klaus Kallenbach, MD, PhD,, Thoralf M. Sundt, MD, and Thomas H. Marwick, MD, PhD, MPH. 

In this paper, it is noted that very few patients were studied (with differing underlying causes of aortic disease), in coming up with the number for surgery, although used to guide the care of millions.  "The number" has generally been coming down over the years as surgery became safer, but the uncertainty of what the number should be remains.

Some individuals suffer aortic tearing, perhaps death, at smaller sizes. On the other hand, surgery has risk, and offering surgery at smaller sizes may injure someone whose aorta would not have torn or ruptured if allowed to grow larger.

 The size of the aneurysm alone is just not enough. Other measures, other ways to understand, are needed, but not available today.

If there were "perfect numbers" for the aorta, our friend Richard would not have dissected at about the same age and the same aneurysm size as my husband (who did not dissect, but had preventive surgery at 5.2 cm). Richard barely survived and has paid a high price in additional surgery because of his original dissection 10 years ago.

For the narrowed aortic valve, aortic stenosis, there are also numbers: the size of the valve opening and the pressure build up. There are measures for the leaking aortic valve also. Are they perfect numbers, that each individual can trust with their life? Is there a promise that nothing bad will happen before you reach the "guideline" numbers? Nothing bad will happen until you have obvious symptoms?

If there were "perfect numbers" for aortic valves, Chuck Doherty would still be with his family. So would  Chad Rogers.

Guidelines, Not Guarantees 

Those with BAV and other forms of aortic disease need to understand there are no guarantees, no solid promises. There are guidelines based on information available today. To our surprise we may find them quite limited, once we understand what they are based upon. As a patient, you need to research for yourself, understanding as much as you can.

Making Decisions
Part of that research can be searching for and choosing physicians/surgeons with a philosophy and approach to risk that is in agreement with your own. There are things to think about, such as your lifestyle, frequency of foreign travel, and general comfort with the risk of surgery versus the risk of waiting. These decisions should be individualized within the context of guidelines and statistics; decisions made in conjunction with physicians whose philosophy and approach to life and risk are in harmony with your own. Physicians who clearly share their outcome statistics with you.

Handling Uncertainty, Owning Decisions
In 2013, Aortic Valve and Ascending Aorta Guidelines for Management and Quality Measures were published. Including references, there are 66 pages. In the summary on page 54 are these words,"The choice of the best procedure or valve is dependent on many factors as discussed above and no procedure or device is ideal. Ultimately it is up to the patient, the cardiologist, and surgeon to reach a decision on appropriate treatment." 

As these guidelines tell us, it is very important that you, the patient, are a partner in these decisions, and that you understand the basis on which you and your physicians together are making these decisions.

Wouldn't it be wonderful if there were perfect numbers, perfect devices, and simple decision making? Yes, but as in much in life, that is rarely the case. There are pros and cons, trade offs, in most decisions that must be made. But we only have one heart, one aorta, so these are very important decisions.

The more you know, the more you understand the pros and cons, you can make decisions that belong to you, that you can own. Not knowing leaves us vulnerable, caught by surprise. Perhaps many of us have already been there, rudely discovering the imperfections and limitations regarding aortic valve options.

Over 20 years ago, the mechanical valve that saved my husband's life was called a lifetime solution. The night I walked into our home and found my fallen husband, terribly injured by that valve and its complications, I began to understand the terrible depths of untruth in that "promise" that we had happily once believed. I will never forget him saying to me, "I did everything I was supposed to do."

If you understand there are no "perfect numbers", no "ideal" devices and solutions, it will at least help you to be proactive, to question, to research, to seek more opinions when things don't make sense, and to partner with your physicians in the decisions that are made.

And when something does not feel right, when an echo or other test results do not make sense, don't let it go. Keep asking questions, seeking answers.

No one cares more than you do.

 Because it is you, the patient,

 above all others,

 who will "own" the outcome.

May this help you to read,

 question,

 and make informed decisions,

while living in a climate of hope.

Best wishes to all,

Arlys Velebir

Bicuspid Aortic Foundation

Escaping Dr. Hodad

Dr. Marty Makary, in his book Unaccountable, writes about Dr. Hodad in the very first chapter. Reading about this doctor, I realized that I had heard of him more than once, in more than one hospital. I just did not know his last name, given to him by surgical residents - HODAD - Hands of Death and Destruction. His name says it all. If you are introduced to him, sign no papers. Escape!Run!

Escaping Death

"I thought you would be dead now."

How could a physician, trusted to protect life and do no harm, look directly at her and say this to Rose? But he did. Who was he? He was not a surgeon. He was a medical doctor. But he was part of the team in a terrible surgical drama that played out in Rose's chest, and he did nothing, nothing at all, to help her.

Yes, Rose fell into the hands of Dr. Hodad and those who collaborated with him. But she did not die. From her story, we can learn why it is worth doing everything possible to avoid the hands of death and destruction in the first place.

Every day, somewhere in the world, someone is having a surgery like Rose needed. There are surgeons who have spent many years perfecting the most complex surgery in the chest, aortic surgery, along with replacing the aortic valve. Rose had been born with BAV, and she also had an ascending aortic aneurysm.

Referred from doctor to doctor in her local city, Rose trusted the doctors she met, trusted them with her life. She believed that she would be referred to the right surgeon, with the right skill. She entered the hospital and had surgery. It was far from ideal, but she came through the complications. With time, she should have been feeling better and better.

Something is Terribly Wrong

But Rose was getting worse. She did not need anyone to tell her that something must be terribly wrong. And it was. Eventually Rose learned that her aortic aneurysm surgery had been botched. Normally a Dacron graft is used to replace the bulging section of aorta that is called an aneurysm. But not in the hands of Dr. Hodad. He had replaced her BAV with a pig's valve, which included part of the pig's aorta above the valve. Dr. Hodad simply cut out Rose's aneurysm, and instead of using Dacron, pulled Rose's own remaining aorta down until it met the pig's above the valve. What went wrong? There was too much tension on her aortic tissue. Rose's stretched aortic tissue began to pull away from the stitches holding it to the pig valve, and blood began to leak out around her valve with every beat of her heart. 

I am amazed at the depths of courage and strength Rose found within her. She fought for her life by researching and reaching out to others, asking questions. She trusted people she did not know. They helped her find her way to an experienced aortic surgeon in another state. She put her life in his experienced hands. Rose's first surgery, in those experienced hands, would have been easy.  This surgery was a fight for life. It took all day. He saved her. 

Escaping Destruction
I remember being told, I think by the anesthesiologist, that the surgeon who would replace my husband's critically narrowed BAV was a good technician. That was over 20 years ago. I didn't appreciate it then as much as I do now. Knowing now how complex that surgery was, I know that the surgeon was at least good enough to get through the challenges - a man in heart failure with a critically narrowed, extremely calcified, and abnormally small BAV. I can imagine a very different outcome with Dr. Hodad. Although, as Dr. Makary writes, the best technician may not be charming like Dr. Hodad, it is important to get the very best skill available to you to save your life.

Years later, my husband had aneurysm surgery. I remember a nurse telling me that his surgeon had "golden hands" and would be her choice. I didn't fully understand the importance of what was said. This was not just being polite. This was someone who worked there and saw the outcomes, day after day. Dr. Makary writes about asking the "insiders" about the doctor you are considering. Those who work there will have more information than you do, if you can find a way to tap into their world.

Finding "Dr. Right"
Often the Bicuspid Aortic Foundation is asked about physicians who can help those with BAV. This is such a difficult dilemma for us all. We do not have enough information to help find Dr. Right in the many places where he is so needed. What we can do is encourage everyone to do everything they can to find Dr. Right and flee from Dr. Hodad.

For those with BAV, medication cannot solve their problems, and surgery and surgeons are extremely important. Thinking about Rose (not her real name), my own family, and the larger genetically related BAV family whose lives all depend on surgical skill, there are things we can do to help ourselves:

• Referrals given may be to Dr. Hodad, not to Dr. Right; you can refer yourself to surgeons in order to find Dr. Right
• Educate yourself about the procedure you need and ask the surgeons you consider many questions 
• Think in terms of the best skill based on results, not the nicest personality 
• Other patients may not know enough about what happened to them; ask the "insiders"

Together,
We are
Creating a 

Climate of Hope

Arlys Velebir

Bicuspid Aortic Foundation

Bicuspid Aortic Valve and Life on the Edge

BJ Sanders at Machu Picchu
7,970 feet above sea level

Those born with  BAV are

 typically high energy adventurers.

BAVers live their lives on the edge!

Yes, the view is fantastic.

But an aortic crisis lies

 just over that edge.  

The challenge is to keep them 

far enough from the edge to be safe,

and still allow them to enjoy

active, vibrant lives.

The problem is that no one knows

exactly where the edge is!

My dear friend, BJ, recently shared the story of her path to surgery 
after 10 years of watching and waiting. 
I have been privileged to walk along with her 

during this time in her life. 

Below I share my side of the story.

 Earlier this Year

BJ, Arlys, Carrie Mettler (l to r)
I love this picture from a BAF
Conference a few years ago!
Both of these ladies are pictures
 of health, and expert at throwing
 curve balls at their doctors!

"Good morning Arlys....Since today is Sunday I hate to bother you....only if you get a chance could you give me a call....if not I will try to catch you on Monday....Thanks...BJ"

Uh, oh! I sensed trouble behind that message and quickly called my friend. She told me that she had experienced major chest pain. Immediately Dr. Raissi helped her lower her blood pressure, and she began to arrange for an imaging study locally. (It shouldn't have been difficult, but just getting the testing became a major challenge! Not a good situation for a BAVer with symptoms!)

BJ was still experiencing episodes of chest pain, and had made plans to come to LA for a consult (prepared to stay for surgery) when I received the following:

"Hi Arlys, 

I wanted to share with you that I had another episode of chest pain. It scares me that it could possibly be aortic pain. Now I am actually afraid. It is a terrible feeling. I have not lived in fear for a decade. I am really grateful to be coming to LA.... Love you, BJ"

Now, her pain was happening at rest, with low blood pressure, once even waking her out of sleep!

Arlys to BJ:

"Just in case have a plan to say the right thing to get help there in case of emergency. I texted Dr. Raissi as want him to know."

Arlys to herself: 

"BJ is complicated - delicate tissue, diabetes, partially paralyzed diaphragm, possible blood clotting issues . . .  She has got to get here for surgery! She will not survive surgery back there, especially an emergency! We all love her, and her family needs her so..." 

Arlys to Dr. Raissi:

"BJ had another pain episode lasted about 12 minutes and bp was "normal".... Wonder if ... room for even lower pressure? could she come here now . . . .?" Dr. Raissi adjusted her medication yet again, driving her blood pressure even lower. The chest pain did not reappear in the short time before her surgery. I believe strictly lowering her blood pressure kept BJ back from that dangerous edge of aortic tearing/rupture until she had surgery. 

How close to that edge did BJ come?

No one really knows.

BJ saw a physician after she returned home who told her that she really was on the "low end" of the numbers that are used to guide the surgery decision. He implied that maybe she didn't really need surgery yet. I mention this because those guidelines are for patients without chest pain. BJ had chest pain, and the numbers don't matter when the aorta begins "talking". This paper from Yale discusses this: Symptoms Plus Family History Trump Size in Thoracic Aortic Aneurysm

Would BJ have fallen over the edge if she had met and listened to this kind of thinking in her search for help? Remember, it is easy to be in denial. When physicians do not recognize aortic pain, perhaps labeling it anxiety, how close to the edge do people come? I have heard of some that fell over. 

Surgery At Last

BJ's BAV

BJ's ascending aneurysm

I arrived very early on the morning of BJ's surgery. We had spoken, but I was anxious to see her. She looked just wonderful, her expressive brown eyes bright and sparkling.  Even I, who know better, marveled at the need for this surgery. She looked so perfectly healthy! Looks are deceiving when it comes to those with BAV. With beautiful skin and vibrant energy, their youthful appearance portrays someone glowing with health. I reminded myself of what was inside her chest, threatening her life. We had to do this!

Pre-Op and Waiting

For years BJ shared the message of Hope with others. 

Now Hope kept her company after surgery!

In pre-op, BJ had her own little cubicle. I was glad for that private time together. She told me she was at peace, and I knew she was. I was so thankful we had safely reached this moment in her journey. We stood on the threshold of the door to her future, engulfed in a Climate of Hope!  

We waited through the hours of her surgery - her husband, Ron, her son, Judd, and I there at the hospital, knowing many others were there in thought with us. BJ has a lovely family, and it was very easy to be there with them. 

Never Trust a Bicuspid!

At some point, "Never Trust a Bicuspid"  has become my motto. Despite efforts to predict what they will do, people with BAV excel in throwing curve balls! Walking in to see BJ that first time after surgery, I was wondering just what surprises she might have in store. 

Dr. Raissi's patients generally look very good after surgery, even in ICU, but I did not quite like my first glimpse of BJ's face. It wasn't long before I knew why, as she threw that first curve. Bright red blood came pouring from her drain tubes! She chose the right time to do this, with her surgeon standing right there to expertly catch it. What would be next?

The next morning, the word initially was good. BJ was off the respirator and sitting up, right on schedule. I decided to wait until afternoon to see her. I should have known better! It wasn't long until I heard from her son, telling me that BJ was going back on the respirator. My heart dropped all the way to my toes!

I had been afraid of problems with BJ's lungs. Some years before, she had been injured during a surgical procedure on her shoulder, resulting in paralysis on one side of her diaphragm. As I drove those miles to the hospital, I tried not to think of the horror stories about respirators and infection.

So what did I find on arrival? Another curve ball! BJ was sitting up, breathing on her own. As long as I have memory, I will never forget that moment. This was one to be thankful for!

Dr Raissi with BJ in ICU

Seconds before they were to put that tube in, BJ somehow turned a corner! Later, Dr. Raissi told us he could see in BJ's eyes that she was better, despite her "numbers", and asked them to wait. Somehow, in that moment physician and patient understood each other, and BJ dodged the respirator! An indescribable moment. 

When we compare memories now, BJ remembers me being there that day, holding her hand. I remember vividly how happy she was to see me walk in. I also remember her saying how tough it had been that morning.  "It was so hard to breathe...."

I waited for more curves -  problems with healing due to her diabetes perhaps. Thankfully that did not materialize. She did manage to find some other bumps on her road to recovery. It was her very own journey, and like many with BAV, she chose her own path! Being under the care of someone used to catching BAV curve balls made all the difference.

I am very grateful as I write this, retracing her BAV journey, that she cleared every hurdle beautifully. I remember watching her walk while still in ICU, and thinking how strong she is. She needed to be.

Who Are these Complex BAVers?

There are variations in those with BAV, and I believe BJ is one of the more complicated. Perhaps what she has deserves its own name, beyond BAV disease, but no one has such a name. The rods that keep her spine straight, her flexible joints, those difficult pregnancies, volatile blood pressure... all tell us her body is special. It concerns me that all these factors may not be taken into consideration in those with BAV. Someday, perhaps, that will change. For now, BJ received the individualized care that her very special body needed. 

How many others need, but do not receive, that care?

In the final few days of 2013, I am happy to share my view of BJ's experience, and to remember with joy my special friend, safely home with her family and friends once again!

Thank you, 

dear BJ,

 for sharing your life 

with us all,

and

Creating a 

Climate of Hope.

      - Arlys Velebir

Journey of the Heart

Dr. Sharo Raissi, Lia Nicandrou, Arlys Velebir, Father Prodromos

At the Bicuspid Aortic Foundation, we are very touched and encouraged whenever we hear from those dealing with BAV and the complications of thoracic aortic disease. We take our mission very seriously, knowing that all around the world we share a common experience with families who have BAV and variations of thoracic aortic disease.

The story we share here, of one young man's journey seeking answers and help, spans the many miles between the island of Cyprus and southern California. It would seem highly unlikely that a young man living in a monastery high in the mountains of Cyprus would find his way to the Bicuspid Aortic Foundation. It would seem even less likely that doors would open that would make it possible for his surgery to be performed here. And critically important for this young man, he would find himself in hands that not only could deal with his ascending aortic aneurysm, but also with the strands of tissue clinging to his 13 year old mechanical valve.

His story, in his own words, is on the BAF website - Journey of the Heart .

We are very grateful that Father Prodromos has so graciously shared his experience with others,

Helping us in our mission, 

and creating a climate of hope,

Arlys Velebir

Chairman and President,

Bicuspid Aortic Foundation

Alicia's Story - From Fatigued and Fainting to a New Life!

Alicia, 3 months after surgery, hiked 3.1 miles!

Her Mom remembers Alicia as a healthy baby. At the age of 7 she developed asthma and was also hospitalized with pneumonia, suffering staff infections. From then on, all was well as she grew and went through her teen age years.

But things changed for her at the age of 20, following the birth of her first child, when she began to have complications.

It was during a routine exam that her doctor heard a heart murmur and suggested she see a cardiologist. At her first cardio exam, she was diagnosed with a bicuspid aortic valve. She was told she was fine and would need to be monitored annually. But she had some difficulties during her pregnancy - dizzy spells and fainting. She gave birth to her daughter in 2009.

Her baby daughter was very ill during her first year of life and was ultimately diagnosed with a PDA (patent ductus arteriosis). At one year of age, the PDA was closed, resulting in a very healthy child ever since.

But for Alicia, all was not well. She tired easily, was short of breath, and often felt faint. When she visited her cardiologist in May of 2011, she was told that nothing had changed.

She was also told that she likely would not need heart surgery until she was in her 40's.

How could she face the next 20 years feeling like this? 

The new year, 2012, found this young mother lightheaded, tiring easily, sometimes fainting, and suffering from migraine head aches. Her cardiologist told her she needed to live with these symptoms, and that she was too young to have surgery. She was also cautioned not to have more children.

The months passed, and the symptoms persisted. Alicia was extremely fatigued and fainted often, also suffering frequent migraines. Her cardiologist prescribed metroprolol for her migraines, which lowered her blood pressure and made her feel worse than ever. Alicia had chronically low blood pressure, and the metroprolol took away what little energy she had.

Their search for help led this family to the Bicuspid Aortic Foundation, looking for information, support, and hope. Alicia sought a second opinion from Dr. Sharo Raissi, who chairs the Foundation's Scientific Advisory Board. She underwent testing that revealed she had an aortic aneurysm along with her BAV. It was clear that Alicia continued to feel unwell, and she could not keep up with her very active toddler. In the fall of 2012, after having further tests, plans were put in place for surgery to remove the aortic aneurysm. The bicuspid aortic valve would be replaced if necessary. Amazingly, in December Alicia completed an extensive pharmacy technician program, despite feeling very tired and weak.

Alicia had surgery on January 16, 2013, at St. John's Health Center. The aneurysm was removed and her bicuspid aortic valve replaced with a bovine prosthetic valve. She now looked forward to feeling better, as her body healed.

By March, she was doing very well indeed. Alicia proved her new found energy and stamina by completing a 3.1 mile hike just three months after surgery (pictured above). What a dream come true! Today she feels great and is so happy. She now knows what it feels like to be the healthy woman she was meant to be! And her daughter notices it too. For the first time in her three years of life, she has a healthy, energetic Mom. This is a very happy family!

Alicia is now well informed about her health. She knows that some day the bovine prosthetic valve will require intervention. In the mean time, she is doing all she can to take charge of her health and her life!

May Alicia's story inspire others to listen to their bodies, and seek the help they need until they find it.

Thank you, Alicia, for sharing your story, and creating a climate of hope.

Paying It Forward - Touching Many Hearts

From One Heart

To Another

"When you helped me, you helped many people......"

Pay It Forward!

Invisible Bonds of the Heart
How do you describe those that have walked together through life and death decisions? I doubt that there is a word in any language. They are connected in a very special way, through an invisible thread between them. Each end of this thread is tied with a very special knot, deep inside their beings. It is a bond that cannot be broken. Over time, as one person helps someone, and they in turn help others, hearts bond together in an unbreakable web. Each one of us can be a part of this wonderful connection. All by paying forward what someone else has done for us.

I have a connection like this to one particular woman in the mid western United States. Her bicuspid aortic valve and aneurysm surgery failed, and only a few months after her surgery she was in serious trouble. It is beyond my comprehension why her physicians did not help her get to a major heart center. Perhaps they thought she was beyond hope. Apparently one of her doctors thought that, because he later told her he expected she would have been dead rather than coming to see him in his office again.

She was not beyond hope! I helped her go to a well known aortic surgeon in another state, and she is not only alive today, she pays the gift of life forward at every opportunity!

Recently, I visited a young man in the hospital. He had just had a much needed surgery. The valve and aneurysm that both threatened him can no longer harm him! He found his way to help not through referrals from physicians, but through his own diligent research and because this special woman reached out to him and pointed the way to help.

This young man is one of several that she has helped. In a recent email, she told me about them. She closed her email this way,

"These are all people who have a chance to live long lives, 

because they are getting expert care.

That's what you taught me, Arlys, that there's care,

 and then there's expert care.

I wouldn't be alive right now, if I hadn't had expert care, so thank you."

You too can pay it forward.

After first getting expert help yourself, share it with another. 

In this way, all around the world

 we join together in this

 indescribable connection of hearts,

Creating a Climate of Hope.

Best wishes,
Arlys Velebir
Bicuspid Aortic Foundation

Great Hearts and Bicuspid Aortic Valves

Dr. Sharo Raissi, Scientific Advisory Board Chairman,  Arlys Velebir, BAF President,
 Dr. Jason Sperling, Scientific Advisory Board Member

Who can help us?

Who do we turn to when a bicuspid aortic valve appears on the scene? Who can help us? It is a question that my family first confronted over 20 years ago.

At that time, my husband was treated in our local medical community, where we first heard the words "bicuspid aortic valve". Other than being told this is a birth defect, nothing in particular was said. My husband received the typical treatment for younger people at that time, replacement with a mechanical valve. We were told he was "fixed" - just take his coumadin (warfarin) faithfully, watch out for infections, and he was set for the rest of his life.

My sister told me about friends of hers back then. The man's aortic valve had been damaged by rheumatic fever. He had it replaced with a mechanical valve and was doing very well. I remember how it comforted me and gave me hope that my husband could be helped too.  

I asked her about this man recently. He still has the same mechanical valve, still takes his anticoagulant, and is doing well. 

That was supposed to happen to my husband too. But it didn't. 

You see, bicuspid aortic valves are "special" and those who have them need special care. As someone from Australia wrote, BAV families have "dodgy" tissue.  The definition of dodgy is very appropriate  - "unsound, unstable, unreliable" and "so risky as to require very deft handling".

Later, when my husband's aneurysm was discovered, we began to understand we were dealing with something "dodgy", and it was going to take some very special help to get through this. Later still, there were more complications.

Great Hearts, Skilled Hands

And so, that is the background, the reason for my smile in the recent picture above, standing between two surgeons who have tremendous hearts and skilled hands, hearts and hands that care for those with BAV. 

I know, so many years later, that is what it takes. Hearts big enough to spend so much time, to give of themselves tirelessly. Spending an hour with you for your first consultation, reading your CT, MRI, and echo  themselves, helping you with blood pressure, answering your questions, and grappling with everything that makes you special. And some day, performing surgery on that dodgy tissue. How dodgy? Well, in my husband's case, it meant taking on yet another, high-risk surgery to deal with pannus and valvular strands on his mechanical valve. Something few have ever heard about, seen, or handled. So far, it has given us an additional 7 precious years together.

What to Look For

At the Foundation we are often asked who can help those with BAV and other forms of Thoracic Aortic Disease (TAD).  It remains a difficult question to answer. Knowledge and technical skill are a must. But there are some other important things to consider.

It should be someone with a heart

 for aortic disease in the chest.

Someone with a heart so large

 that they generously give their time and talents.

Someone that truly cares for you and your family. 

Someone skilled enough

and fearless enough

 to take on your "dodgy" tissue. 

Captured in the picture above,

I was privileged to stand between

two such great hearts.

Standing there,

I knew I was surrounded 

by a climate of hope,
enveloped in

an atmosphere of caring.

May you find the same.

Arlys Velebir

President, Bicuspid Aortic Foundation

.

Victory Over Aortic Aneurysm - the OTHER KILLER in the Chest

Something Is Terribly Wrong!

Sandra on Barker Mountain, 2012

Sandra Croff knew something was wrong, but no one had been able to find it. She had tremendous fatigue, shortness of breath, chest pain, and at times felt her heart doing flip flops. 

After seemingly endless doctor visits and tests, she was told that her heart was fine. She was just suffering from post menopausal symptoms and anxiety.

Among those many tests, Sandra had a simple chest x-ray. She remembers looking at it with the doctor, and asking him "What is that?" When told it was her aorta, she asked the doctor if it should be so large. The doctor dismissed it, saying her aorta was fine, and proceeded to tell her she must have fibromyalgia. Sandra firmly told the doctor, "I do not." She turned and left that office, disappointed again. 

Sandra remembers one particular day very well. She had horrible pain on the left side of her chest, going through to her back, and into her arm. It lasted for about three hours. Sandra did not go to the ER that day, because based on past experience she felt no one there would believe or help her. But she knew she had to keep looking for answers, because others in her family had died suddenly, supposedly of massive heart attacks.  She wondered if her own life would be cut short before she found anyone to help her. And she also wondered, even if she lived, if she would ever feel well enough to do even simple things she loved again without labored breathing and a stabbing, burning feeling in her chest. 

Sandra, 7 days after aortic surgery

In 2010, Sandra made a call to a long-time family friend. She had heard that he had some kind of heart surgery, and she wanted to know the details. That family friend was Bob Gies, and before long Sandra traveled to the same aortic treatment program. There she had the testing she needed. It showed that Sandra had an aortic aneurysm in her chest. She began to make plans for surgery.

Something is Now Very Right!
Sandra now has her life back! Always active, Sandra proved that her problems were not related to declining hormones, anxiety, or fibromyalgia, but due to faulty "plumbing" in her chest. Below, in her own words, Sandra describes hiking Barker Mountain recently.

Hiking Barker Mountain, 2011

Hiking Barker Mountain, September 2012, in Sandra's Own Words

I was in Montana this week and hiked to the top of Barker Mountain again. The day started out at a cool 42 degrees, and some of the rooftops had frost on them.  My hike began at the bottom of the mountain, just where the road splits and Green Creek crosses.  Arriving at the starting point, I had to get my boots on, so that I could embark on this steep outing. It was about 11:30 am as I started to walk and also take pictures of the new survey, being done by the Forest Service. 

As I crossed Galena Creek a couple of times, I could feel the steep grade tasking my body, not only my lungs but my legs.  I felt my heart beating faster and louder.  I am from a part of the country that is only about 150' above sea level.  These people in Montana are like mountain goats, as far as their lungs and their capability to walk this steep terrain!  I guess I am a flat lander? When I reached one stretch that is much steeper than the rest, I would stop briefly and have a drink of water.  

Much further up the mountain, there is a long stretch where the cool breeze comes down the narrow roadway.  It refreshed my mind, and also rejuvenated my body.  The breeze along with the sweet smell of the pines is just beyond description.  It is the type of breeze that makes you smile with pleasure.  Oh, how I love the breeze!  As a child I lived in Southern California, and in the morning it would be cool to chilly, and the breeze would come from the Pacific Ocean to energize you. The last point where you feel this breeze on the mountain is situated just below where the sun hits the side hill.  From that point on you are in the sun completely.  Knowing this is the last pull to get to the top, I am telling myself, "You are almost there!" and "Get moving!". 

After getting to the top, a large rock was the perfect place for lunch.  I was out of steam at that point! After eating and drinking water, it was time to start the descent. Of course this is the easiest part of the hike, however the ground has unstable rock on it and care is needed to avoid sliding.

It was indeed a body and mind builder.  On the way down, a drink of fresh, cool water from Daisy Creek was indescribable.  This small creek comes from higher up the mountain and is the best water around.  It is very cold, and thirst-quenching. 

This was the second time I have hiked this mountain since I had my aortic surgery. 
Of course, being 64 years does not say you will be able to do everything you desire but your mind allows you to achieve what you want, especially if your heart is fit. It did my heart good to make this hike again this year. As I was driving down the road, I was able to see the mountain in my rear view mirror, and all I could think of was next year's hike!

"In Our Hands" - Outcomes of Surgery

"In Our Hands"

When surgeons speak about their results, they often use the phrase "in our hands", graciously crediting the entire surgical team. 

Their patients think a great deal about hands also, more specifically about one pair of hands, the hands of the surgeon. 

We wonder if surgeons realize how much we look at their hands when we meet them. Our minds are racing - are these the right hands to open my chest, touch my heart, replace my valve, remove my bulging aorta? Can I trust these hands?

Practice Makes Perfect
Common sayings are rooted in practical life experience, and the phrase "practice makes perfect" is no exception. The more we do something, the better we get!

Outcomes of Aortic Valve Replacement Surgery
In 2003, the New England Journal of Medicine published a special article, Surgeon Volume and Operative Mortality in the United States.  It was a report on the outcomes of 8 surgical procedures in Medicare patients. One of  those procedures, aortic valve replacement, is important to those with aortic disease. There was further discussion about it in this article also, High surgical volume equals better patient outcomes. Looking at a chart in the NE Journal article, (Table 2), the affect on outcome of the hospital's volume for aortic valve replacement was 0%. The affect of the surgeon's volume on outcome was 100%. 

In other words, it was all about the surgeon's hands.  

Asking Questions

That is why it is so important to ask certain questions of the surgeon. Make a list! How many bicuspid (or trileaflet) aortic valves have you replaced (or repaired) this year? Last year? Last five years?  How many aortic aneurym procedures? How many of this exact procedure did you do this year ( last year, last five years, etc.)? (Bypass results are not of interest unless bypass surgery is needed, so be careful to avoid general totals of all heart surgery.) How many people died in the hospital? How many died within 30 days? How many were injured or had complications? What were they? How many had infection? Bleeding? How many needed permanent pacemakers after surgery? How many needed heart medications long term that they did not need before surgery? The Foundation Support Page has additional information that may be helpful.

Listening Carefully to the Answers

The Foundation has heard from some patients who believe they did not receive clear, straight forward answers. Clear communication can be difficult in many settings, but especially so when talking about your own major surgery. Take someone with you. 

For example, if you ask how long a certain valve has lasted in others, listen carefully for general answers such as "I have never had to take one out." Follow up with more questions. Find out if the surgeon is talking about a clinical trial that has been going on for a few months, or a valve that has been used for many years with an established track record. That background can put a whole new meaning around the word "never".

Do your best to avoid things like these that have happened to others:

• A woman did not realize she was part of a clinical trial until the hospital contacted her months later for a "free" echo of her prosthetic aortic valve.
• A man arrived home after surgery thinking he had received a certain kind of tissue valve. He had specifically discussed and requested that kind of tissue valve when talking with the surgeon. He was more than a little surprised to discover later that in his chest was a different kind of tissue valve, from a different manufacturer. No one had told him while he was in the hospital. 
• A man, still in the hospital, was shocked when the technician told him during his echo that he had a beautiful mechanical valve. He and his family were sure he had requested tissue. No one had told him anything about receiving a mechanical valve until the technician made that comment. If there were technical reasons for the mechanical, no one had explained it to him or his family.
• A woman thought that her ascending aortic aneurysm was replaced with Dacron. When she began to have problems a few months later, in reading her surgery report she discovered that Dacron was not used at all. Her aorta had simply been "shortened". It put her in life-threatening jeopardy as the stitches holding her stretched aorta pulled away from  a newly implanted valve.
• A man who had previously had his bicuspid aortic valve replaced required another surgery for a large ascending aortic aneurysm. When opening his chest, his aorta was punctured, causing massive, uncontrolled bleeding. He survived the crisis, but after surgery contracted numerous infections. After fighting for many weeks in the hospital, he lost his battle.

Would it have been different for them in other hands? It is an important question to ponder. 

Surgeon's Statistics

Statistics may be available if you search for them. Some states provide a public database of surgical results, however those results may only be for the most common heart surgery performed, bypass surgery. Search and see what you find.

Grateful for Pioneering Hands

Dr. Randall Griepp and Dr. Sharo Raissi

Dr. Randall Griepp, Dr. Teruhisa Kazui, Dr. Sharo Raissi

The phrase "in our hands" takes on special meaning as we think of the great pioneers in aortic surgery.  Dr. Randall Griepp for many years has advanced treatment of the aorta in the chest. His hands have pioneered many lifesaving procedures, taking on the most difficult challenges in the chest from the early days of heart transplants to circulatory arrest and the aorta.

Under his guidance, Mount Sinai developed a rich data resource of results over the years, forming the basis of many important, highly regarded papers on thoracic aortic disease.



The Foundation is grateful for Dr. Griepp's work, and that of other aortic surgeons we have honored, Dr. Nicholas Kouchoukos, and Dr. Teruhisa Kazui. Their work has saved many lives around the world.

Arlys Velebir and Dr. Nicholas Kouchoukos

When it comes time for surgery, may we all do our best to find expert, skilled hands in which to entrust our lives.

Choosing your Doctors and Hospitals

 

Give a man a fish, and he will eat for a day.

We Have Something in Common
In some ways we are alike, in others we are different. But we all have something special in common in our chests - aortic valve disease, aortic aneurysm, aortic dissection. Sometimes, we have brain aneurysms too.

And so, we have something else in common.

 Decisions about who to entrust with our medical care.

Critical decisions.

Too critical to rely on a simple list of names. 

My Family Needs Help! Who Is the Best?
Like most people, we turned to local physicians when my husband became ill the first time. We learned that he was in heart failure due to a severely narrowed bicuspid aortic valve. At that time, we had no way of researching our options.

We simply went from the internist to the cardiologist, the cardiologist to the surgeon. You may find it hard to believe, but we did not even meet the man who would open his chest and touch my husband's heart until the night before surgery, already admitted to the hospital.

Years later, the internet had emerged when the aneurysm was discovered in his chest. This time I knew there was no one in the local community equipped to understand and help. But there was still very limited information. There were just scattered crumbs along the way that we used to reach the hands of a skilled aortic expert.

As I write this in 2012, we still do not have all the tools we need to help families like my own research aortic disease in the chest. But we do have some - and we can use them to find help.

Researching Your Options Learning to Fish!
The Foundation is often asked about finding physicians with expertise in aortic disease in the chest. Until recently there have not been many options - just a handful of major centers in the US pioneered aortic surgery over the years, while heart surgeries in most communities were bypass surgery, along with some heart valves.

When it came to bicuspid aortic valves, it was considered simple, something that did not need specialized attention. That is just beginning to change.

This is where the fishing lessons come in - we all need to learn how to find the medical expertise that can help ourselves and our families. It will continue to evolve, new expertise and capabilities will emerge. How will we know who to trust with our lives? It is not as simple as a list of names. We need to do our homework. The Bicuspid Aortic Foundation has updated it's  Support Community Page - there are some ideas there to help you!

                             Teach a man to fish, and he will eat for a lifetime.                    

Bob Gies Learned to Fish and You Can Too!
Look carefully and you will see the fish on Bob's line in those clear Montana waters! When he began to have symptoms, Bob visited several cardiologist and surgeon offices, looking for help, not satisfied with the answers.

Today, he has both local care and follow up as needed with a specialized bicuspid aortic valve program. But he had to search to find it!

Bob has care not just for one day, but for a lifetime.

He knows how to be an active partner in his own health.

When Bob approached the Bicuspid Aortic Foundation, he did not just receive a fish for one day. He learned to fish for a life time. Today, he volunteers with the Foundation, reaching out to others. He knows they too can learn to fish!

This is an important topic for us all. Please check back for additional future stories to help you in your search.

Best wishes from my family to you and yours,
Arlys Velebir
President and Chairman
Bicuspid Aortic Foundation